7 Things to Know About Primary Biliary Cholangitis

By

Sarah Handzel, BSN, RN

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Primary biliary cholangitis (PBC), also known as primary biliary cirrhosis, is a type of liver disease that can lead to serious complications, like liver failure. PBC results from the destruction of bile ducts in your liver. These ducts, or tubes, allow bile to flow from your liver into your small intestine, where it helps with digestion and allows your body to rid itself of excess toxins, cholesterol, and damaged red blood cells. As more of your bile ducts are destroyed, more scar tissue forms within your liver. This severely impacts your liver’s ability to function properly.

Fortunately, we have a good understanding of PBC and how it can be treated. There are several things you should know about PBC, but your doctor can give you a better understanding of your personal therapy goals and prognosis.

1. PBC is often discovered accidently.

PBC progresses slowly over time, and many people are unaware they have PBC until it’s discovered accidently. Your doctor may first suspect PBC due to information gathered from routine blood tests that check your liver function.

If you doctor thinks you have PBC, he or she will order further tests, including an anti-mitochondrial antibody (AMA) blood test. These types of antibodies, or proteins, almost never occur in healthy individuals; therefore, if your AMA test is positive, there’s a high likelihood that you have PBC. In some cases, liver biopsies are performed to confirm a diagnosis.

2. PBC may be an autoimmune disorder.

Recent research suggests PBC may be caused, in part, by an overactive immune system. Some research shows certain types of immune system cells, called T lymphocytes (T cells), collect in the liver. Once there, they destroy healthy bile duct cells, causing inflammation and scarring.

3. There may be a hereditary link.

The exact cause of PBC is unknown, but research indicates a hereditary component to the disease. People with parents or siblings, especially an identical twin sibling, with PBC are more likely to develop the condition. This suggests specific genetic components or mutations may be responsible for causing this condition.

4. Certain groups are more at risk.

Anyone can get PBC, but it’s more common in women compared to men. Individuals between the ages of 30 and 60 are more likely to develop PBC. Additionally, this liver disease occurs most often in people living in North America or Europe.

5. PBC usually develops slowly.

PBC usually develops slowly over a period of many years. It can take up to 20 years for symptoms to start. Fortunately, modern medications can help slow the progression of this disease even further, helping you to live a healthier life for a longer period of time.

6. Symptoms can be severe.

Symptoms can be especially severe once they occur. Common symptoms of primary biliary cholangitis include:

  • Dry mouth and eyes

  • Fatigue

  • Itchy skin

  • Pain in the abdomen, bones, or joints

  • Buildup of fluid in the abdomen, legs, and feet

  • Jaundice, or yellowing of the eyes and skin

  • Skin darkening

  • Osteoporosis, or weak, brittle bones

  • High cholesterol

  • Mental status changes

  • Weight loss

7. There is no cure.

There is no cure for PBC. But certain medications provide tremendous benefits by slowing the progression of the disease so you can live a longer, healthier life. If you have PBC, your doctor will likely recommend treatment with ursodeoxycholic acid (UDCA), which helps bile move through your liver and into your small intestine. Other medications, such as obeticholic acid (Ocaliva) and fibrates are used to improve liver function and decrease the severity of some symptoms, like itching.

Even though the cause of PBC isn’t known, our understanding of the condition has already lead to great improvements in treatment and outcomes for people living with the disease. If you’re concerned about your personal outlook and treatment plan, a conversation with your doctor can lead to a better understanding of why specific recommendations were made for you. Working together with your healthcare team, you can help slow the progression of PBC and live a healthy life for as long as possible.

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Medical Reviewers: William C. Lloyd III, MD, FACS Last Review Date: Jan 19, 2018

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View Sources

Medical References

  1. Primary Biliary Cholangitis. American Liver Foundation. https://www.liverfoundation.org/for-patients/about-the-liver/diseases-of-the-liver/primary-biliary-cholangitis/#support-groups
  2. Primary biliary cholangitis. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis-pbc/symptoms-causes/syc-20376874
  3. Primary biliary cholangitis (primary biliary cirrhosis). National Institute of Diabetes and Digestive and Kidney Diseases. https://www.niddk.nih.gov/health-information/liver-disease/primary-biliary-cholangitis
  4. Primary Biliary Cholangitis. National Organization for Rare Disorders. https://rarediseases.org/rare-diseases/primary-biliary-cholangitis/
  5. Primary Biliary Cirrhosis (PBC). Merck Manual Professional Version. http://www.merckmanuals.com/professional/hepatic-and-biliary-disorders/fibrosis-and-cirrhosis/primary-biliary-cirrhosis-pbc
  6. Primary biliary cholangitis. Genetic and Rare Diseases Information Center. https://rarediseases.info.nih.gov/diseases/7459/primary-biliary-cholangitis

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